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While the advancement of perioperative management has expanded Fontan candidacy, not all patients have a successful postoperative course. Our case was a right isomerism patient who could not leave the ICU due to high central venous pressure and low output syndrome. Initial observation of the monitor ECG showed his rhythm to be supraventricular, however, an echocardiogram indicated simultaneous contraction of the atrium and ventricle, implying a junctional rhythm. While neither central venous pressure nor blood pressure improved with temporary pacing, better central venous and pulmonary venous blood flow patterns during pacing unraveled its positive impact. The patient successfully left the ICU after permanent pacing implantation. Hemodynamic study revealed a beneficial impact of atrial pacing in securing cardiac output and ventricular preload, lowering central venous pressure, and shortening blood transit time, which is partly attributed to the optimization of the fenestration function in reservation of the preload. Our case emphasizes the significant advantage of atrial pacing in a failing Fontan patient with junctional rhythm by reducing venous congestion and maximizing the benefit of fenestration.

We present the case of a 1-year-old boy who developed protein-losing enteropathy (PLE) within 2 months of a fenestrated Fontan procedure. His fenestration rapidly closed despite bilateral pulmonary stenosis (BPS). Subsequent to PLE onset, both fenestration and the bilateral pulmonary artery were reconstructed, and the patient's PLE had been in remission, with additive use of medications, for more than 2 years. Notably, although fenestration closed again and central venous pressure (CVP) reduction was minimal, the surrogates of venous return resistance were markedly suppressed as shown by increased blood volume, reduced estimated mean circulatory filling pressure, and suppressed CVP augmentation against a contrast agent. Taken together, dynamic characteristics of venous stagnation, rather than the absolute value of CVP, were ameliorated by the pulmonary reconstruction and use of medications, suggesting a significant role of venous property in the physiology of PLE. In addition, simultaneous measures of CVP and ventricular end-diastolic pressure during the abdominal compression procedure suggested a limited therapeutic role of fenestration against PLE in this patient.

In this study, we tested our hypothesis that thyroid function is impaired and contributes to perturbed hemodynamics in patients after Fontan operation. Cardiac catheterization and blood tests for thyroid function were performed in 37 patients who underwent a Fontan operation. Among them, 12 patients (33%) had subclinical thyroid dysfunction with an elevated thyroid-stimulating hormone level despite normal thyroxine levels. Thyroid-stimulating hormone levels were significantly correlated with central venous pressure (p <0.01, R 2  = 0.3), and patients with subclinical hypothyroidism showed significantly elevated γ-glutamyltransferase level, an indicator of liver congestion, compared with the other patients (125.6 ± 12.2 vs 67.6 ± 4.6 IU/L, p <0.01). In addition, the levels of free triiodothyronine, an effective thyroid hormone, were significantly lower in patients with subclinical hypothyroidism than in those with normal thyroid function (3.1 ± 0.1 vs 3.5 ± 0.1 pg/dl, p <0.01). The free triiodothyronine level was significantly and negatively correlated with the relaxation time constant (p = 0.03) and brain natriuretic hormone (p <0.01) level and positively correlated with the cardiac index (p = 0.04). In conclusion, venous congestion in Fontan patients may cause thyroid dysfunction, which can be responsible for decreased ventricular function and cardiac output in Fontan patients. Thus, thyroid function should be routinely monitored after Fontan surgery., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: In the Fontan circulation, driving forces with respiration, heartbeat, and lower limb muscle pump are relevant. However, the mechanics of these forces has not been proven, and their effects on the Fontan circulation remain unclear., Methods: We performed catheter examinations and measured pressure and flow velocity simultaneously in the bilateral pulmonary arteries of 12 Fontan patients 1 year after the operation. The pulmonary pressure and flow velocity data were decomposed into respiratory and heartbeat components by discrete Fourier analysis. We then calculated respiratory and cardiac wave intensity (WI) based on the respiratory and heartbeat components of pressure and flow velocity data., Results: Respiratory WI formed 2 negative peaks, a backward expansion wave during the inspiratory phase, and then a backward compression wave during the expiratory phase. In 2 phrenic nerve palsy cases and 1 case of a patient on a respirator, respiratory WI showed disturbed patterns and a negative pattern, respectively. Cardiac WI showed 2 or 4 negative peaks, the time phase of which matched that of the atrial contractions., Conclusions: WI analysis elucidated that inspiration acts as a sucking driving force and increases the pulmonary blood flow in the Fontan circulation. Respiratory complications compromise efficiency in the Fontan circulation. It was also revealed that the pulmonary blood flow was mutually dammed up and sucked in by increases and decreases in atrial pressure., (Copyright © 2016 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Flow energy loss (EL) at the Fontan anastomosis has been thought to reflect flow efficiencies and to influence on hemodynamics in the Fontan circulation and has been often discussed in numerical studies. However, in vivo EL measurements have to date not been reported. We directly measured EL in the Fontan circulation and examined the relationship between the structural configuration and EL, as well as the influence of EL, on the hemodynamics in the Fontan circulation. We performed a catheterization study measuring simultaneous pressure and flow velocity to calculate EL in nine patients (mean age 2.3 ± 0.3 years) 1 year after the Fontan procedure. The measured EL was 9.66 ± 8.50 mW. One patient with left pulmonary artery stenosis recorded the highest EL (17.78 mW), and one patient with bilateral superior vena cava and left phrenic nerve palsy recorded the second highest EL (14.62 mW). EL significantly correlated with time constant tau and weakly with max-dp/dt during the isovolumic diastolic phase (r = 0.795 and -0.574, respectively). EL also correlated with max-dp/dt during the isovolumic systolic phase (r = 0.842) but not with ejection fraction or systemic blood flow (r = 0.384 and -0.034, respectively). In conclusion, inefficient structural configuration and phrenic nerve palsy seem to be related with increased in EL at the Fontan anastomosis. Although these preliminary findings also suggest that EL is associated with an impaired relaxation of the ventricle, a long-term study with a large population is warranted to reach such a definitive conclusion.